BACKGROUND: The median age of diagnosis of primary sclerosing cholangitis (PSC) is ∼30-40 years. OBJECTIVE: We aimed to analyse disease progression and liver-dependent survival in patients diagnosed with PSC after 50 years of age. METHODS: Patients with PSC were analysed with regard to their age at diagnosis. Patients with a first diagnosis of PSC after the age of 50 years were considered as the late-onset group. RESULTS: A total of 32/215 (14.9%) patients were diagnosed with PSC after 50 years of age. The proportion of females was significantly higher among patients with late-onset PSC (48.4 vs. 27.3%; p = 0.02). Patients with later diagnosis required dilatation therapy more often due to dominant stenosis (84.2 vs. 53.1%; p = 0.01) and suffered from recurrent cholangitis more often (48.3 vs. 21.0%; p = 0.003). Patients with late-onset PSC had reduced transplantation-free survival (10.5 ± 0.6 years vs. 20.8 ± 1.7 years, p < 0.0001), with progredient liver failure and cholangiocarcinoma as the leading causes of death. CONCLUSIONS: Patients with later age at diagnosis of PSC displayed a different clinical phenotype with a different sex ratio, immune status and an increased risk for progressive liver failure and biliary malignancies.
BACKGROUND: The median age of diagnosis of primary sclerosing cholangitis (PSC) is ∼30-40 years. OBJECTIVE: We aimed to analyse disease progression and liver-dependent survival in patients diagnosed with PSC after 50 years of age. METHODS: Patients with PSC were analysed with regard to their age at diagnosis. Patients with a first diagnosis of PSC after the age of 50 years were considered as the late-onset group. RESULTS: A total of 32/215 (14.9%) patients were diagnosed with PSC after 50 years of age. The proportion of females was significantly higher among patients with late-onset PSC (48.4 vs. 27.3%; p = 0.02). Patients with later diagnosis required dilatation therapy more often due to dominant stenosis (84.2 vs. 53.1%; p = 0.01) and suffered from recurrent cholangitis more often (48.3 vs. 21.0%; p = 0.003). Patients with late-onset PSC had reduced transplantation-free survival (10.5 ± 0.6 years vs. 20.8 ± 1.7 years, p < 0.0001), with progredient liver failure and cholangiocarcinoma as the leading causes of death. CONCLUSIONS: Patients with later age at diagnosis of PSC displayed a different clinical phenotype with a different sex ratio, immune status and an increased risk for progressive liver failure and biliary malignancies.
Authors: Tobias J Weismüller; Palak J Trivedi; Annika Bergquist; Mohamad Imam; Henrike Lenzen; Cyriel Y Ponsioen; Kristian Holm; Daniel Gotthardt; Martti A Färkkilä; Hanns-Ulrich Marschall; Douglas Thorburn; Rinse K Weersma; Johan Fevery; Tobias Mueller; Olivier Chazouillères; Kornelius Schulze; Konstantinos N Lazaridis; Sven Almer; Stephen P Pereira; Cynthia Levy; Andrew Mason; Sigrid Naess; Christopher L Bowlus; Annarosa Floreani; Emina Halilbasic; Kidist K Yimam; Piotr Milkiewicz; Ulrich Beuers; Dep K Huynh; Albert Pares; Christine N Manser; George N Dalekos; Bertus Eksteen; Pietro Invernizzi; Christoph P Berg; Gabi I Kirchner; Christoph Sarrazin; Vincent Zimmer; Luca Fabris; Felix Braun; Marco Marzioni; Brian D Juran; Karouk Said; Christian Rupp; Kalle Jokelainen; Maria Benito de Valle; Francesca Saffioti; Angela Cheung; Michael Trauner; Christoph Schramm; Roger W Chapman; Tom H Karlsen; Erik Schrumpf; Christian P Strassburg; Michael P Manns; Keith D Lindor; Gideon M Hirschfield; Bettina E Hansen; Kirsten M Boberg Journal: Gastroenterology Date: 2017-03-06 Impact factor: 22.682
Authors: Kirsten Boonstra; Rinse K Weersma; Karel J van Erpecum; Erik A Rauws; B W Marcel Spanier; Alexander C Poen; Karin M van Nieuwkerk; Joost P Drenth; Ben J Witteman; Hans A Tuynman; Anton H Naber; Paul J Kingma; Henk R van Buuren; Bart van Hoek; Frank P Vleggaar; Nan van Geloven; Ulrich Beuers; Cyriel Y Ponsioen Journal: Hepatology Date: 2013-10-17 Impact factor: 17.425
Authors: Ramon V Cortez; Luana N Moreira; Marina Padilha; Mariana D Bibas; Ricardo K Toma; Gilda Porta; Carla R Taddei Journal: Front Immunol Date: 2021-02-05 Impact factor: 7.561